A. Ablasser, I. Hemmerling, J. L. Schmid-burgk, R. Behrendt, A. Roers et al., TREX1 deficiency triggers cell-autonomous immunity in a cGAS-dependent manner, J. Immunol, vol.192, pp.5993-5997, 2014.

M. Amor-guéret, Bloom syndrome, genomic instability and cancer: the SOS-like hypothesis, Cancer Lett, vol.236, pp.1-12, 2006.

H. Babbe, N. Chester, P. Leder, and B. Reizis, The Bloom's syndrome helicase is critical for development and function of the alphabeta T-cell lineage, Mol. Cell. Biol, vol.27, pp.1947-1959, 2007.

H. Babbe, J. Mcmenamin, E. Hobeika, J. Wang, S. J. Rodig et al., Genomic instability resulting from Blm deficiency compromises development, maintenance, and function of the B cell lineage, J. Immunol, vol.182, pp.347-360, 2009.

D. V. Bugreev, X. Yu, E. H. Egelman, and A. V. Mazin, Novel pro-and anti-recombination activities of the Bloom's syndrome helicase, Genes Dev, vol.21, pp.3085-3094, 2007.

B. S. Carvalho and R. A. Irizarry, A framework for oligonucleotide microarray preprocessing, Bioinformatics, vol.26, pp.2363-2367, 2010.

S. Cerboni, N. Jeremiah, M. Gentili, U. Gehrmann, C. Conrad et al., Intrinsic antiproliferative activity of the innate sensor STING in T lymphocytes, J. Exp. Med, vol.214, pp.1769-1785, 2017.

P. Chabosseau, G. Buhagiar-labarchède, R. Onclercq-delic, S. Lambert, M. Debatisse et al., Pyrimidine pool imbalance induced by BLM helicase deficiency contributes to genetic instability in Bloom syndrome, Nat. Commun, vol.2, p.368, 2011.

K. L. Chan and I. D. Hickson, New insights into the formation and resolution of ultra-fine anaphase bridges, Semin. Cell Dev. Biol, vol.22, pp.906-912, 2011.

K. L. Chan, P. S. North, and I. D. Hickson, BLM is required for faithful chromosome segregation and its localization defines a class of ultrafine anaphase bridges, EMBO J, vol.26, pp.3397-3409, 2007.

Q. Chen, L. Sun, and Z. J. Chen, Regulation and function of the cGAS-STING pathway of cytosolic DNA sensing, Nat. Immunol, vol.17, pp.1142-1149, 2016.

N. Chester, F. Kuo, C. Kozak, C. D. O'hara, and P. Leder, Stage-specific apoptosis, developmental delay, and embryonic lethality in mice homozygous for a targeted disruption in the murine Bloom's syndrome gene, Genes Dev, vol.12, pp.3382-3393, 1998.

F. Coquel, M. J. Silva, H. Técher, K. Zadorozhny, S. Sharma et al., SAMHD1 acts at stalled replication forks to prevent interferon induction, Nature, vol.557, pp.57-61, 2018.
URL : https://hal.archives-ouvertes.fr/hal-01791237

Y. J. Crow and N. Manel, Aicardi-Goutières syndrome and the type I interferonopathies, Nat. Rev. Immunol, vol.15, pp.429-440, 2015.

C. Cunniff, J. A. Bassetti, and N. A. Ellis, Bloom's syndrome: clinical spectrum, molecular pathogenesis, and cancer predisposition, Mol. Syndromol, vol.8, pp.4-23, 2017.

Z. Dou, K. Ghosh, M. G. Vizioli, J. Zhu, P. Sen et al., Cytoplasmic chromatin triggers inflammation in senescence and cancer, Nature, vol.550, pp.402-406, 2017.

S. Dutertre, M. Ababou, R. Onclercq, J. Delic, B. Chatton et al., Cell cycle regulation of the endogenous wild type Bloom's syndrome DNA helicase, Oncogene, vol.19, pp.2731-2738, 2000.

S. Eladad, T. Z. Ye, P. Hu, M. Leversha, S. Beresten et al., Intra-nuclear trafficking of the BLM helicase to DNA damage-induced foci is regulated by SUMO modification, Hum. Mol. Genet, vol.14, pp.1351-1365, 2005.

N. A. Ellis, J. Groden, T. Z. Ye, J. Straughen, D. J. Lennon et al., The Bloom's syndrome gene product is homologous to RecQ helicases, Cell, vol.83, pp.90105-90106, 1995.

E. Erdal, S. Haider, J. Rehwinkel, A. L. Harris, and P. J. Mchugh, A prosurvival DNA damage-induced cytoplasmic interferon response is mediated by end resection factors and is limited by Trex1, Genes Dev, vol.31, pp.353-369, 2017.

F. Foucault, C. Vaury, A. Barakat, D. Thibout, P. Planchon et al., Characterization of a new BLM mutation associated with a topoisomerase II alpha defect in a patient with Bloom's syndrome, Hum. Mol. Genet, vol.6, pp.1427-1434, 1997.

D. Gao, J. Wu, Y. T. Wu, F. Du, C. Aroh et al., Cyclic GMP-AMP synthase is an innate immune sensor of HIV and other retroviruses, Science, vol.341, pp.903-906, 2013.

S. Gemble, A. Ahuja, G. Buhagiar-labarchède, R. Onclercq-delic, J. Dairou et al., Pyrimidine pool disequilibrium induced by a cytidine deaminase deficiency inhibits PARP-1 activity, leading to the under replication of DNA, PLoS Genet, vol.11, 2015.

M. Gentili, J. Kowal, M. Tkach, T. Satoh, X. Lahaye et al., Transmission of innate immune signaling by packaging of cGAMP in viral particles, Science, vol.349, pp.1232-1236, 2015.

J. German, Bloom's syndrome, Dermatol. Clin, vol.13, issue.18, pp.30101-30104, 1995.

S. Glück, B. Guey, M. F. Gulen, K. Wolter, T. W. Kang et al., Innate immune sensing of cytosolic chromatin fragments through cGAS promotes senescence, Nat. Cell Biol, vol.19, pp.1061-1070, 2017.

K. H. Goss, M. A. Risinger, J. J. Kordich, M. M. Sanz, J. E. Straughen et al., Enhanced tumor formation in mice heterozygous for Blm mutation, Science, vol.297, pp.2051-2053, 2002.

D. J. Gough, N. L. Messina, C. J. Clarke, R. W. Johnstone, and D. E. Levy, Constitutive type I interferon modulates homeostatic balance through tonic signaling, Immunity, vol.36, pp.166-174, 2012.

E. Gul, E. H. Sayar, B. Gungor, F. K. Eroglu, N. Surucu et al., Type I IFN-related NETosis in ataxia telangiectasia and Artemis deficiency, J. Allergy Clin. Immunol, vol.142, pp.246-257, 2018.

S. M. Harding, J. L. Benci, J. Irianto, D. E. Discher, A. J. Minn et al., Mitotic progression following DNA damage enables pattern recognition within micronuclei, Nature, vol.548, pp.466-470, 2017.

G. , Journal of Experimental Medicine 1211 BLM prevents innate sensing of micronuclei by cGAS

A. Härtlova, S. F. Erttmann, F. A. Raffi, A. M. Schmalz, U. Resch et al., DNA damage primes the type I interferon system via the cytosolic DNA sensor STING to promote anti-microbial innate immunity, Immunity, vol.42, pp.332-343, 2015.

T. H. Hütteroth, S. D. Litwin, and J. German, Abnormal immune responses of Bloom's syndrome lymphocytes in vitro, J. Clin. Invest, vol.56, pp.1-7, 1975.

E. M. Jenkinson, M. P. Rodero, P. R. Kasher, C. Uggenti, A. Oojageer et al., Mutations in SNORD118 cause the cerebral microangiopathy leukoencephalopathy with calcifications and cysts, Nat. Genet, vol.48, pp.1185-1192, 2016.

W. J. Kaiser, J. L. Kaufman, and M. K. Offermann, IFN-alpha sensitizes human umbilical vein endothelial cells to apoptosis induced by doublestranded RNA, J. Immunol, vol.172, pp.1699-1710, 2004.

J. K. Karow, A. Constantinou, J. L. Li, S. C. West, and I. D. Hickson, The Bloom's syndrome gene product promotes branch migration of holliday junctions, Proc. Natl. Acad. Sci. USA, vol.97, pp.6504-6508, 2000.

A. Kauffmann, R. Gentleman, and W. Huber, arrayQualityMetrics-a bioconductor package for quality assessment of microarray data, vol.25, pp.415-416, 2009.

N. Kondo, F. Motoyoshi, S. Mori, N. Kuwabara, T. Orii et al., Long-term study of the immunodeficiency of Bloom's syndrome, Acta Paediatr, vol.81, pp.86-90, 1992.

X. Lahaye, T. Satoh, M. Gentili, S. Cerboni, C. Conrad et al., The capsids of HIV-1 and HIV-2 determine immune detection of the viral cDNA by the innate sensor cGAS in dendritic cells, Immunity, vol.39, pp.1132-1142, 2013.
URL : https://hal.archives-ouvertes.fr/inserm-00959028

L. Bennani-belhaj, K. , S. Rouzeau, G. Buhagiar-labarchède, P. Chabosseau et al., The Bloom syndrome protein limits the lethality associated with RAD51 deficiency, Mol. Cancer Res, vol.8, pp.385-394, 2010.

Y. Y. Lan, D. Londoño, R. Bouley, M. S. Rooney, and N. Hacohen, Dnase2a deficiency uncovers lysosomal clearance of damaged nuclear DNA via autophagy, Cell Reports, vol.9, pp.180-192, 2014.

T. Li and Z. J. Chen, The cGAS-cGAMP-STING pathway connects DNA damage to inflammation, senescence, and cancer, J. Exp. Med, vol.215, pp.1287-1299, 2018.

G. Luo, I. M. Santoro, L. D. Mcdaniel, I. Nishijima, M. Mills et al., Cancer predisposition caused by elevated mitotic recombination in Bloom mice, Nat. Genet, vol.26, pp.424-429, 2000.

K. J. Mackenzie, P. Carroll, C. A. Martin, O. Murina, A. Fluteau et al., cGAS surveillance of micronuclei links genome instability to innate immunity, Nature, vol.548, pp.461-465, 2017.

N. Manel, B. Hogstad, Y. Wang, D. E. Levy, D. Unutmaz et al., A cryptic sensor for HIV-1 activates antiviral innate immunity in dendritic cells, Nature, vol.467, pp.214-217, 2010.

I. Meyts and J. L. Casanova, A human inborn error connects the ?'s, Nat. Immunol, vol.17, pp.472-474, 2016.

G. H. Nguyen, W. Tang, A. I. Robles, R. P. Beyer, L. T. Gray et al., Regulation of gene expression by the BLM helicase correlates with the presence of G-quadruplex DNA motifs, Proc. Natl. Acad. Sci. USA, vol.111, pp.9905-9910, 2014.

N. Nicolas, D. Moshous, M. Cavazzana-calvo, D. Papadopoulo, R. De-chasseval et al., A human severe combined immunodeficiency (SCID) condition with increased sensitivity to ionizing radiations and impaired V(D)J rearrangements defines a new DNA recombination/repair deficiency, J. Exp. Med, vol.188, pp.627-634, 1998.

C. Poinsignon, D. Moshous, I. Callebaut, R. De-chasseval, I. Villey et al., The metallo-beta-lactamase/beta-CASP domain of Artemis constitutes the catalytic core for V(D)J recombination, J. Exp. Med, vol.199, pp.315-321, 2004.

H. Quek, J. Luff, K. Cheung, S. Kozlov, M. Gatei et al., Rats with a missense mutation in Atm display neuroinflammation and neurodegeneration subsequent to accumulation of cytosolic DNA following unrepaired DNA damage, J. Leukoc. Biol, vol.101, pp.4-0716, 2017.

M. Raab, M. Gentili, H. Belly, H. R. Thiam, P. Vargas et al., ESCRT III repairs nuclear envelope ruptures during cell migration to limit DNA damage and cell death, Science, vol.352, pp.359-362, 2016.

C. Ralf, I. D. Hickson, and L. Wu, The Bloom's syndrome helicase can promote the regression of a model replication fork, J. Biol. Chem, vol.281, pp.22839-22846, 2006.

V. A. Rao, C. Conti, J. Guirouilh-barbat, A. Nakamura, Z. H. Miao et al., Endogenous gamma-H2AX-ATM-Chk2 checkpoint activation in Bloom's syndrome helicase deficient cells is related to DNA replication arrested forks, Mol. Cancer Res, vol.5, pp.713-724, 2007.

G. I. Rice, G. M. Forte, M. Szynkiewicz, D. S. Chase, A. Aeby et al., Assessment of interferon-related biomarkers in Aicardi-Goutières syndrome associated with mutations in TREX1, RNASEH2A, RNA-SEH2B, RNASEH2C, SAMHD1, and ADAR: a case-control study, Lancet Neurol, vol.12, pp.1159-1169, 2013.

M. E. Ritchie, B. Phipson, D. Wu, Y. Hu, C. W. Law et al., limma powers differential expression analyses for RNAsequencing and microarray studies, Nucleic Acids Res, vol.43, 2015.

M. P. Rodero, J. Decalf, V. Bondet, D. Hunt, G. I. Rice et al., Detection of interferon alpha protein reveals differential levels and cellular sources in disease, J. Exp. Med, vol.214, pp.1547-1555, 2017.
URL : https://hal.archives-ouvertes.fr/pasteur-01534181

M. P. Rodero, A. Tesser, E. Bartok, G. I. Rice, E. D. Mina et al., Type I interferonmediated autoinflammation due to DNase II deficiency, Nat. Commun, vol.8, p.2176, 2017.
URL : https://hal.archives-ouvertes.fr/pasteur-01768950

A. Rongvaux, R. Jackson, C. C. Harman, T. Li, A. P. West et al., Apoptotic caspases prevent the induction of type I interferons by mitochondrial DNA, Cell, vol.159, pp.1563-1577, 2014.

N. E. Sanjana, O. Shalem, and F. Zhang, Improved vectors and genomewide libraries for CRISPR screening, Nat. Methods, vol.11, pp.783-784, 2014.

J. W. Schoggins, S. J. Wilson, M. Panis, M. Y. Murphy, C. T. Jones et al., A diverse range of gene products are effectors of the type I interferon antiviral response, Nature, vol.472, pp.481-485, 2011.

D. B. Stetson, J. S. Ko, T. Heidmann, and R. Medzhitov, Trex1 prevents cell-intrinsic initiation of autoimmunity, Cell, vol.134, pp.587-598, 2008.

A. Subramanian, P. Tamayo, V. K. Mootha, S. Mukherjee, B. L. Ebert et al., Gene set enrichment analysis: a knowledge-based approach for interpreting genome-wide expression profiles, Proc. Natl. Acad. Sci. USA, vol.102, pp.15545-15550, 2005.

L. Sun, J. Wu, F. Du, X. Chen, and Z. J. Chen, Cyclic GMP-AMP synthase is a cytosolic DNA sensor that activates the type I interferon pathway, Science, vol.339, pp.786-791, 2013.

J. Tissari, J. Sirén, S. Meri, I. Julkunen, and S. Matikainen, IFN-alpha enhances TLR3-mediated antiviral cytokine expression in human endothelial and epithelial cells by up-regulating TLR3 expression, J. Immunol, vol.174, pp.4289-4294, 2005.

M. Tohyama, X. Dai, K. Sayama, K. Yamasaki, Y. Shirakata et al., dsRNA-mediated innate immunity of epidermal keratinocytes, Biochem. Biophys. Res. Commun, vol.335, pp.505-511, 2005.

C. W. Van-kerckhove, J. L. Ceuppens, M. Vanderschueren-lodeweyckx, E. Eggermont, S. Vertessen et al., Bloom's syndrome: clinical features and immunologic abnormalities of four patients, Am. J. Dis. Child, vol.142, pp.1089-1093, 1988.

A. P. West, W. Khoury-hanold, M. Staron, M. C. Tal, C. M. Pineda et al., Mitochondrial DNA stress primes the antiviral innate immune response, Nature, vol.520, pp.553-557, 2015.

G. , Journal of Experimental Medicine 1212 BLM prevents innate sensing of micronuclei by cGAS

M. J. White, K. Mcarthur, D. Metcalf, R. M. Lane, J. C. Cambier et al., Apoptotic caspases suppress mtDNA-induced STING-mediated type I IFN production, Cell, vol.159, pp.1549-1562, 2014.

S. R. Woo, M. B. Fuertes, L. Corrales, S. Spranger, M. J. Furdyna et al., STING-dependent cytosolic DNA sensing mediates innate immune recognition of immunogenic tumors, Immunity, vol.41, pp.830-842, 2014.

L. Wu and I. D. Hickson, The Bloom's syndrome helicase suppresses crossing over during homologous recombination, Nature, vol.426, pp.870-874, 2003.

H. Yang, H. Wang, J. Ren, Q. Chen, and Z. J. Chen, 2017. cGAS is essential for cellular senescence, Proc. Natl. Acad. Sci. USA, vol.114, pp.4612-4620
URL : https://hal.archives-ouvertes.fr/hal-02097449

V. Yankiwski, R. A. Marciniak, L. Guarente, and N. F. Neff, Nuclear structure in normal and Bloom syndrome cells, Proc. Natl. Acad. Sci. USA, vol.97, pp.5214-5219, 2000.

Q. Yu, Y. V. Katlinskaya, C. J. Carbone, B. Zhao, K. V. Katlinski et al., DNA-damage-induced type I interferon promotes senescence and inhibits stem cell function, Cell Reports, vol.11, pp.785-797, 2015.

G. , Journal of Experimental Medicine 1213 BLM prevents innate sensing of micronuclei by cGAS